Clinical characteristics and outcome in the acute phase of ischemic locked-in syndrome: case series of twenty patients with ischemic LIS.

Background: Locked-in syndrome (LIS) is a condition characterized by quadriplegia and anarthria. The most common cause is a ventral pontine lesion due to atherosclerotic basilar artery disease. Methods: Cases with LIS were prospectively identified among the patients with acute ischemic stroke over 3 years, between 2009 and 2011. Clinical characteristics, topographic localization of lesions, and outcome were determined during the first 6 months from onset of LIS. Results: Our case series consists of 20 patients (mean age 62 8 10 years; range 46-82). Initially 16 patients had a reduced level of consciousness (mean 3 days; range 1-15). Respiratory disturbance, mainly as impairment of the breathing pattern, was noted in all cases. Five patients died within the first 10 days due to stroke progression or cardiac arrest. In the remaining cases the most frequent causes of death were pulmonary infections and sepsis. Overall mortality in the acute phase of LIS is 75%, and the median survival time is 42 days. There was a statistically significant association between the more extensive parenchymal brain stem lesions and observed mortality. Conclusions: Ischemic LIS is commonly caused by an acute complete occlusion of the basilar artery due to atherosclerotic lesions in intracranial vertebrobasilar vessels. Mortality remains high in the acute phase of the disease. Copyright (C) 2013 S. Karger AG, Basel